Disclosures: Nanichi A. Ramos Roldan, MD: No financial relationships or conflicts of interest

Case Diagnosis: Movement disorder as a presentation of antiphospholipid syndrome.

Case Description: A 79 year old male with history of migraines with aura, hypothyroidism, and multiple pulmonary embolisms presented with involuntary movements, initially affecting left upper and lower extremities, significantly impairing his ability to function. Symptoms progressively worsened over 8 months to involve the left side of mouth. On examination, the high amplitude chorea-like movements involved violent flinging of left hemibody suggestive of hemiballismus.

Setting: Acute Tertiary HospitalAssessment/

Results: Patient underwent a comprehensive workup with brain MRI remarkable for bilateral T1 hyperintensities in basal ganglia, but with noted improvement when compared to previous imaging, despite worsening of symptoms. His paraneoplastic workup was negative, however, further workup revealed an elevated cardiolipin IgM and beta-2 glycoprotein IgM, which was confirmed on repeat testing two weeks later. Given history of multiple pulmonary embolisms and elevated antiphospholipid antibodies, he was diagnosed with antiphospholipid syndrome (APS). These findings favor that his chorea was likely secondary to APS, as other causes were excluded. He was treated with IVIG, methylprednisolone and clonazepam without improvement, such that he was ultimately started on aripiprazole.

Discussion: Antiphospholipid syndrome rarely presents with movement disorders, but can be seen in 1-4% of patients. Movement disorders can present long before other features of APS, with chorea being the most common, including oromandibular chorea. Nevertheless, of the chorea presentations, hemiballismus is the rarest. In cases where APS is the cause of the chorea, aripiprazole has been proven to reduce symptoms.

Conclusion: When evaluating the cause of chorea, it is important to keep a broad differential diagnosis. Currently, there is no diagnostic criteria for APS-related chorea. In patient with APS, the presence of choreiform movements is suggestive of this condition. A careful clinical assessment, exclusion of other neurological conditions, combined with laboratory results and radiologic imaging may exclude the other causes of chorea.

Level of Evidence: Level V

« Back to AAPM&R Annual Assembly 2021

PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/chorea-as-an-atypical-presentation-of-antiphospholipid-syndrome-a-case-report/